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Genetic absence epilepsy in rats from Strasbourg — A review

  • Conference paper
Generalized Non-Convulsive Epilepsy: Focus on GABA-B Receptors

Part of the book series: Journal of Neural Transmission ((NEURAL SUPPL,volume 35))

Summary

We have selected a strain of rats and designated it the Genetic Absence Epilepsy Rat from Strasbourg (GAERS). In this strain, 100% of the animals present recurrent generalized non-convulsive seizures characterized by bilateral and synchronous spike-and-wave discharges accompanied with behavioural arrest, staring and sometimes twitching of the vibrissae. Spontaneous SWD (7–11 cps, 300–1,000 µV, 0.5–75 sec) start and end abruptly on a normal background EEG. They usually occur at a mean frequency of 1.5 per min when the animals are in a state of quiet wakefulness. Drugs effective against absence seizures in humans (ethosuccimide, trimethadione, valproate, benzodiazepines) suppress the SWD dose-dependently, whereas drugs specific for convulsive or focal seizures (carbamazepine, phenytoin) are ineffective. SWD are increased by epileptogenic drugs inducing petit mal-like seizures, such as pentylenetetrazol, gamma-hydroxybutyrate, THIP and penicillin.

Depth EEG recordings and lesion experiments show that SWD in GAERs depend on cortical and thalamic structures with a possible rhythmic triggering by the lateral thalamus. Most neurotransmitters are involved in the control of SWD (dopamine, noradrenaline, NMDA, acetylcholine), but GABA and gamma-hydroxybutyrate (GHB) seem to play a critical role. SWD are genetically determined with an autosomal dominant inheritance. The variable expression of SWD in offsprings from GAERS × control reciprocal crosses may be due to the existence of multiple genes.

Neurophysiological, behavioural, pharmacological and genetic studies demonstrate that spontaneous SWD in GAERS fulfil all the requirements for an experimental model of absence epilepsy. As the mechanisms underlying absence epilepsy in humans are still unknown, the analysis of the genetic thalamocortical dysfunction in GAERS may be fruitful in investigations of the pathogenesis of generalized non-convulsive seizures.

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Authors and Affiliations

  1. Service de Neurologie I, C.H.U., Strasbourg, France

    Dr. C. Marescaux

  2. Laboratoire de Neurophysiologie et Biologie des Comportements, Centre de Neurochimie, C.N.R.S., Strasbourg, France

    M. Vergnes & A. Depaulis

  3. Service de Neurologie I, Hôpital Universitaire, 1 place de l’Hôpital, F-67091, Strasbourg Cedex, France

    Dr. C. Marescaux

Authors
  1. Dr. C. Marescaux
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  2. M. Vergnes
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  3. A. Depaulis
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Editors and Affiliations

  1. Clinique Neurologique, Hôpital Civil, Strasbourg, France

    C. Marescaux

  2. Centre de Neurochimie, C.N.R.S., Strasbourg, France

    M. Vergnes

  3. Pharma Division, Ciba-Geigy AG, Basel, Switzerland

    R. Bernasconi

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© 1992 Springer-Verlag

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Marescaux, C., Vergnes, M., Depaulis, A. (1992). Genetic absence epilepsy in rats from Strasbourg — A review. In: Marescaux, C., Vergnes, M., Bernasconi, R. (eds) Generalized Non-Convulsive Epilepsy: Focus on GABA-B Receptors. Journal of Neural Transmission, vol 35. Springer, Vienna. https://doi.org/10.1007/978-3-7091-9206-1_4

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  • DOI: https://doi.org/10.1007/978-3-7091-9206-1_4

  • Publisher Name: Springer, Vienna

  • Print ISBN: 978-3-211-82340-8

  • Online ISBN: 978-3-7091-9206-1

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